CAY10550

Cellular prion protein (PrP^c) is a cell surface glycoprotein expressed in brain, spinal cord, and several peripheral tissues that if mutated to the protease-resistant isoform PrP^Sc (scrapie PrP, PrP^res) can result in one of several fatal neurodegenerative diseases.¹ Prion diseases, including mad cow disease (bovine spongiform encephalopathy), scrapie, and Creutzfeldt-Jakob disease develop from the accumulation of PrP^Sc, an abnormally folded β-rich conformation of PrP^c.^2,3 CAY10550 is a potent antiprion compound that inhibits the accumulation of PrP^c with an IC₅₀ value of 3 nM in both ScN2a and F3 prion-infected mouse neuroblastoma cell lines.⁴ This compound also demonstrates moderate radical scavenging activity as it inhibits the formation of hydroxyl radicals in vitro with an IC₅₀ value of 90 µM.⁴

WARNING This product is not for human or veterinary use.

1. Prusiner, S.B. Shattuck lecture-neurodegenerative diseases and prions. N. Engl. J. Med. 344(20), 1516-1526 (2001).

2. Stahl, N., and Prusiner, S.B. Prions and prion proteins. The FASEB Journal 5, 2799-2807 (1991).

3. Prusiner, S.B. Prions. Proc. Natl. Acad. Sci. USA 95, 13363-13383 (1998).

4. Kimata, A., Nakagawa, H., Ohyama, R., et al. New series of antiprion compounds: Pyrazolone derivatives have the potent activity of inhibiting protease-resistant prion protein accumulation. J. Med. Chem. 50, 5053-5056 (2007).